Anemia and Sickle Cell Disease
Types of anemia, and managing sickle cell crises
| Anemia | Clues | Treatment and teaching |
|---|---|---|
| Iron deficiency | Small, pale cells (microcytic); fatigue, pallor, spoon-shaped nails, pica (craving ice or dirt); often from blood loss or pregnancy | Oral iron on an empty stomach if tolerated, with vitamin C; not with milk, antacids or tea; every-other-day dosing may absorb better. Dark stools and constipation are expected. Liquid iron through a straw (stains teeth). IV iron for intolerance |
| Vitamin B12 (including pernicious anemia) | Large cells (macrocytic); numbness, tingling, poor balance, memory problems, smooth red tongue | B12 injections or high-dose oral B12; lifelong in pernicious anemia (missing intrinsic factor) |
| Folate deficiency | Large cells without nerve symptoms; alcohol use, pregnancy, poor diet | Folic acid; leafy greens, legumes, fortified grains |
| Anemia of CKD | Low erythropoietin | Iron + erythropoiesis-stimulating agents; don't push Hgb too high (stroke, clots) |
| Aplastic anemia | Bone marrow failure: low red cells, white cells and platelets | Infection and bleeding precautions; transfusions; stem cell transplant |
Most stable patients are transfused when Hgb <7 g/dL (often <8 with heart disease or surgery).
Sickle cell disease
Inherited (autosomal recessive). Red cells sickle when stressed → block small vessels and break down early.
Crisis triggers
Dehydration, infection, cold, low oxygen (high altitude), stress, overexertion.
Vaso-occlusive pain crisis
- Treat pain fast (aim within 1 hour of arrival): IV opioids, often by PCA; scheduled, not just "as needed".
- Hydration (oral and IV).
- Oxygen only if SpO₂ is low.
- Warmth: no cold packs (cold causes vessel narrowing).
- Incentive spirometry to prevent acute chest syndrome.
Emergencies
- Acute chest syndrome: chest pain, fever, cough, low SpO₂, new lung infiltrate: leading cause of death.
- Stroke (children: yearly transcranial Doppler screening).
- Splenic sequestration (young children): sudden big spleen, pallor, shock.
- Fever ≥101.3°F (38.5°C), or per protocol: urgent evaluation (no working spleen → sepsis risk).
Long-term Updated
- Hydroxyurea (raises fetal hemoglobin; check blood counts; avoid in pregnancy), L-glutamine, crizanlizumab.
- Voxelotor was withdrawn from the market in 2024.
- Gene therapies (exa-cel, lovo-cel) approved in 2023 for ages ≥12.
- Children: daily penicillin until age 5; pneumococcal and meningococcal vaccines.